Primary Pyruvate Dehydrogenase Complex Deficiency Overview
2021年6月17日 · Primary pyruvate dehydrogenase complex deficiency (PDCD) is a mitochondrial disorder of carbohydrate oxidation that mostly affects the brain and leads to decreased ATP …
丙酮酸脱氢酶的结构、功能与相关疾病 - 知乎 - 知乎专栏
丙酮酸彻底氧化分解需要先转变为乙酰辅酶A，由 丙酮酸脱氢酶复合体 （pyruvate dehydrogenase complex，PDH或PDC）催化。 这个反应需要三种酶连续催化，依次称为E1（丙酮酸脱氢 …
Pyruvate Dehydrogenase Complex Deficiency - ScienceDirect
In pyruvate dehydrogenase complex (PDHC) deficiency, energy production from carbohydrate metabolism is severely reduced, and excess pyruvate is converted to lactate. The most …
Pyruvate dehydrogenase deficiency disease detected by the …
2021年6月22日 · Pyruvate dehydrogenase complex (PDHC) deficiency is a common neurodegenerative disease associated with abnormal mitochondrial energy metabolism. The …
Pyruvate dehydrogenase complex deficiency is linked to …
2018年8月1日 · Mutations such as that leading to the αV138M variant in pyruvate dehydrogenase, the pyruvate-decarboxylating PDHc E1 component, can result in PDHc …
Pyruvate dehydrogenase complex deficiency and its relationship …
2015年10月1日 · The pyruvate dehydrogenase complex (PDHc) is a member of a family of multienzyme complexes that provides the link between glycolysis and the tricarboxylic acid …
Pyruvate dehydrogenase complex deficiency and its …
PDHc deficiency is a metabolic disorder commonly associated with lactic acidosis, progressive neurological and neuromuscular degeneration that vary with age and gender. In this review, …
丙酮酸脱氢酶复合体的研究进展及应用-【维普期刊官网】- 中文期 …
摘要 综述了丙酮酸脱氢酶复合体 (PDHc)的组成、结构功能、调控机制、缺陷疾病及在医学和农业中的应用.丙酸酸脱氢酶系是定位在线粒体中的多酶复合体,它是由丙酮酸脱氢酶 (E1)、二氢硫 …
Pyruvate dehydrogenase deficiencies - SpringerLink
The extraordinary complexity of the pyruvate dehydrogenase complex (PDHC) is clearly documented in Chapter 12. PDHC contains three catalytic and two regulatory enzymes. It is …
基础医学院张兴/常云杰团队Nature Communications揭示哺乳动物 …
本研究利用冷冻电子断层扫描技术（Cryo-Electron Tomography, Cryo-ET），首次揭示了哺乳动物完整PDHc的原位三维结构，详细分析了外周亚基E1和E3的数量与空间排布，提供了PDHc高 …